Epidemiologic study of major complications in adolescent and adult patients with thalassemia in Northeastern Thailand: the E-SAAN study phase I

Teawtrakul, Nattiya; Jetsrisuparb, Arunee; Pongudom, Saranya; Chansung, Kanchana; Wanitpongpun, Chinadol; Fucharoen, Supan

doi:10.1080/10245332.2017.1358845

Cited by 29 publications

(27 citation statements)

References 38 publications

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“…We have also documented a Thai β-thalassemia intermedia patient with inferior vena cava thrombosis [18]. A recent epidemiological study on the major complications in TDT and non-TDT of adolescent and adult patients in the region has confirmed this [19]. The study found that 11.1% of non-TDT patients were those with Hb H disease who had evidence of thrombosis and leg ulcer.…”

Section: Discussionsupporting

confidence: 57%

Elevations of Thrombotic Biomarkers in Hemoglobin H Disease

Chansai

Fucharoen

et al. 2018

Acta Haematol

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Background: Thalassemia is a group of hereditary hemoglobinopathies caused by decreased or absent synthesis of α and/or β globin chains. Studies have shown that hypercoagulability and thrombosis are common clinical symptoms in β-thalassemia, especially β-thalassemia intermedia, but little is known about in α-thalassemia. This study aims to examine phosphatidylserine (PS) levels, platelet activation, and coagulation markers in splenectomized (S) and nonsplenectomy (NS) patients with hemoglobin (Hb) H disease. Methods: The NS group comprised 20 patients (median age 15.0 years, range, 14–16.5 years), and the S group consisted of 11 patients (median age 16.4 years, range, 14–19.9 years) with Hb H disease; the control group consisted of 20 normal subjects. Hematological parameters were collected. Flow cytometry was used to measure PS exposure on red blood cells. The levels of intercellular adhesive molecule (ICAM)-1, tumor necrosis factor α (TNFα), β-thromboglobulin (TG) and prothrombin fragment 1 + 2 (F1.2) were determined using ELISA test kits. Results: Significant increases in the levels of PS, ICAM-1, TNFα, β-TG, and F1.2 were observed in both patient groups compared to normal controls (p < 0.01). Conclusion: This observation indicates blood coagulation, endothelial injury, chronic low-grade inflammation, platelet activation, and thrombin generation are present in Hb H disease; these findings merit further assessment in a larger prospective cohort to establish possible links with thrombotic manifestations.

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Section: Discussionsupporting

confidence: 57%

Elevations of Thrombotic Biomarkers in Hemoglobin H Disease

Chansai

Fucharoen

et al. 2018

Acta Haematol

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“…β-thalassaemia. A better understanding about the risk factors for major complications of the disease, including PH, has been derived from significant cohorts [69]. Furthermore, D erchi et al .…”

Section: Updated Clinical Classification Of Phmentioning

confidence: 99%

“…Following the first association of splenectomy and PH [74], it was not clear whether it represented a risk factor or a particular condition. Since then, splenectomy has been associated with the development of PH in many haematological conditions, as in the aforementioned β-thalassaemia [69, 70]. Most importantly, it has been strongly associated with the development of chronic thromboembolic PH [75].…”

Section: Updated Clinical Classification Of Phmentioning

confidence: 99%

Haemodynamic definitions and updated clinical classification of pulmonary hypertension

et al. 2019

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Since the 1st World Symposium on Pulmonary Hypertension (WSPH) in 1973, pulmonary hypertension (PH) has been arbitrarily defined as mean pulmonary arterial pressure (mPAP) ≥25 mmHg at rest, measured by right heart catheterisation. Recent data from normal subjects has shown that normal mPAP was 14.0±3.3 mmHg. Two standard deviations above this mean value would suggest mPAP >20 mmHg as above the upper limit of normal (above the 97.5th percentile). This definition is no longer arbitrary, but based on a scientific approach. However, this abnormal elevation of mPAP is not sufficient to define pulmonary vascular disease as it can be due to an increase in cardiac output or pulmonary arterial wedge pressure. Thus, this 6th WSPH Task Force proposes to include pulmonary vascular resistance ≥3 Wood Units in the definition of all forms of pre-capillary PH associated with mPAP >20 mmHg. Prospective trials are required to determine whether this PH population might benefit from specific management.Regarding clinical classification, the main Task Force changes were the inclusion in group 1 of a subgroup “pulmonary arterial hypertension (PAH) long-term responders to calcium channel blockers”, due to the specific prognostic and management of these patients, and a subgroup “PAH with overt features of venous/capillaries (pulmonary veno-occlusive disease/pulmonary capillary haemangiomatosis) involvement”, due to evidence suggesting a continuum between arterial, capillary and vein involvement in PAH.

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“…The complications related to iron overloading in patients with transfusion include myocardiopathy, congestive heart failure, liver cirrhosis, arthritis, endocrine disorders such as diabetes, and other diseases. In recent years, the prevalence of abnormal glucose metabolism in β -thalassemia major has gradually increased, which may be linked to increased life expectancy in patients with β -thalassemia major and growing concern about the combination of thalassemia with diabetes [4, 5]. However, according to previously published studies, the prevalence of abnormal glucose metabolism in β -thalassemia major was controversial.…”

Section: Introductionmentioning

confidence: 99%

Elevated Prevalence of Abnormal Glucose Metabolism and Other Endocrine Disorders in Patients withβ-Thalassemia Major: A Meta-Analysis

Cui

Yang

et al. 2019

BioMed Research International

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Background. Endocrinopathies are common in patients withβ-thalassemia major despite parenteral iron chelation therapy with deferoxamine. Prevalence of abnormal glucose metabolism in previous studies was controversial. The aim of this study was to discuss the prevalence of abnormal glucose metabolism inβ-thalassemia major based on a meta-analysis.Methods. PubMed, ScienceDirect, Springerlink, Ovid, Web of Science, MEDLINE, Wanfang database, and Chinese National Knowledge Internet were searched for relevant articles. Two authors selected the articles according to the inclusion criteria and then extracted the data. The prevalence of diabetes mellitus (DM) inβ-thalassemia major was defined as the primary outcome. The prevalence with the 95% confidence interval (95%CI) was used to evaluate the proportion of abnormal glucose metabolism and other endocrine disorders in patients withβ-thalassemia major. Subgroup analyses were applied to explore the prevalence in different regions. Sensitivity analysis and publication bias assessment were also conducted.Results. A total of 44 studies with 16605 cases were included in this analysis. Diabetes mellitus was present in 6.54% (95% CI: 5.30%-7.78%). The fixed subgroup study revealed that the region with the highest prevalence was the Middle East (prevalence= 7.90%, 95% CI: 5.75%-10.05%). The accumulated meta-analysis revealed that the prevalence of DM inβ-thalassemia major was relatively steady in each year. The prevalence of impaired fasting glucose (IFG), impaired glucose tolerance (IGT), and other endocrine disorders inβ-thalassemia major was 17.21% (95% CI: 8.43%-26.00%), 12.46% (95% CI: 5.98%-18.94%), and 43.92% (95% CI: 37.94%-49.89%), respectively. Sensitivity analysis showed that the pooled results were robust; publication bias assessment revealed that there was no significant evidence that the pooled results were influenced by publication bias.Conclusion. High prevalence of endocrine disorders involving abnormal glucose metabolism was detected inβ-thalassemia major. Treatment and prevention measurements may be necessary to prevent growth and endocrine problems.

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Epidemiologic study of major complications in adolescent and adult patients with thalassemia in Northeastern Thailand: the E-SAAN study phase I

Abstract: Nearly half of the patients in this cohort had disease-related complications. Splenectomy and advanced age were important factors for complication involvement. Early screening for the complications may reduce the morbidity and mortality in patients with thalassemia.

Cited by 29 publications

References 38 publications

Elevations of Thrombotic Biomarkers in Hemoglobin H Disease

Elevations of Thrombotic Biomarkers in Hemoglobin H Disease

Haemodynamic definitions and updated clinical classification of pulmonary hypertension

Elevated Prevalence of Abnormal Glucose Metabolism and Other Endocrine Disorders in Patients withβ-Thalassemia Major: A Meta-Analysis

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